β-hCG secreting uterine PEComa

A postmenopausal female patient presented with vaginal bleeding. Initial bloodwork revealed an elevated serum beta human chorionic gonadotropin level (β-hCG). Pelvic MRI identified a complex heterogeneous uterine mass with central necrosis. She underwent total abdominal hysterectomy with bilateral saplingo-oopherectomy. Pathology reported a malignant perivascular epithelioid cell tumour (PEComa). Postoperatively, her β-hCG level returned to normal. β-hCG secreting sarcomas are extremely rare, and to our knowledge, there has only been one previously reported case of a β-hCG secreting PEComa. Based on the limited literature, these tumours may have a worse prognosis. The role of β-hCG as a marker of treatment response and disease activity is unclear. Additional studies are required to further ascertain its role as a predictive and prognostic biomarker.